Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case Series
In 2012, a new neoplasm was classified according to fusion of the B-cell lymphoma (BCL-6) corepressor (BCOR) gene and the testis-specific cyclin B3 (CCNB3) gene on the X-chromosome, known as a BCOR-CCNB3 fusion positive sarcoma. Traditionally, BCOR sarcomas have been classified as 'Ewing-like&#...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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The Korean Society of Pediatric Hematology-Oncology
2022-10-01
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| Series: | Clinical Pediatric Hematology-Oncology |
| Subjects: | |
| Online Access: | https://doi.org/10.15264/cpho.2022.29.2.60 |
| _version_ | 1811198806656548864 |
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| author | Salma Omar Karen Albritton Kenneth Heym Jason Wang Anish Ray |
| author_facet | Salma Omar Karen Albritton Kenneth Heym Jason Wang Anish Ray |
| author_sort | Salma Omar |
| collection | DOAJ |
| description | In 2012, a new neoplasm was classified according to fusion of the B-cell lymphoma (BCL-6) corepressor (BCOR) gene and the testis-specific cyclin B3 (CCNB3) gene on the X-chromosome, known as a BCOR-CCNB3 fusion positive sarcoma. Traditionally, BCOR sarcomas have been classified as 'Ewing-like' due to similarities in morphology. However, BCOR-CCNB3 fusion positive sarcomas are molecularly and genetically distinct. Previous studies have focused on clinical and pathologic characterization of this specific malignancy, but standard treatment modalities are not well documented. We present three pediatric patients diagnosed with BCOR-CCNB3 sarcomas. A two-year-old girl and a 16-year-old boy were treated using a five-drug therapy consisting of vincristine, doxorubicin, etoposide, ifosfamide, and cyclophosphamide. A 12-yearold girl received a two-drug therapy using a combination of ifosfamide and doxorubicin. All three patients are in remission following chemotherapy and surgery, confirming the effectiveness and safety of the outlined regimens. There is a lack of consensus regarding an appropriate therapy algorithm for Ewing sarcoma patients with BCOR. The present study adds to the extant literature by detailing effective, yet varying treatment modalities. |
| first_indexed | 2024-04-12T01:37:29Z |
| format | Article |
| id | doaj.art-9c5cbeeb672d4376a492a6009240cc27 |
| institution | Directory Open Access Journal |
| issn | 2233-5250 |
| language | English |
| last_indexed | 2024-04-12T01:37:29Z |
| publishDate | 2022-10-01 |
| publisher | The Korean Society of Pediatric Hematology-Oncology |
| record_format | Article |
| series | Clinical Pediatric Hematology-Oncology |
| spelling | doaj.art-9c5cbeeb672d4376a492a6009240cc272022-12-22T03:53:17ZengThe Korean Society of Pediatric Hematology-OncologyClinical Pediatric Hematology-Oncology2233-52502022-10-01292606410.15264/cpho.2022.29.2.60cpho.2022.29.2.60Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case SeriesSalma Omar0Karen Albritton1Kenneth Heym2Jason Wang3Anish Ray4Texas College of Osteopathic Medicine, University of North Texas Health Science Center, TX, USADepartment of Pediatric Hematology/Oncology, Cook Children’s Health Care System, Fort Worth, TX, USADepartment of Pediatric Hematology/Oncology, Cook Children’s Health Care System, Fort Worth, TX, USADepartment of Pediatric Hematology/Oncology, Cook Children’s Health Care System, Fort Worth, TX, USADepartment of Pediatric Hematology/Oncology, Cook Children’s Health Care System, Fort Worth, TX, USAIn 2012, a new neoplasm was classified according to fusion of the B-cell lymphoma (BCL-6) corepressor (BCOR) gene and the testis-specific cyclin B3 (CCNB3) gene on the X-chromosome, known as a BCOR-CCNB3 fusion positive sarcoma. Traditionally, BCOR sarcomas have been classified as 'Ewing-like' due to similarities in morphology. However, BCOR-CCNB3 fusion positive sarcomas are molecularly and genetically distinct. Previous studies have focused on clinical and pathologic characterization of this specific malignancy, but standard treatment modalities are not well documented. We present three pediatric patients diagnosed with BCOR-CCNB3 sarcomas. A two-year-old girl and a 16-year-old boy were treated using a five-drug therapy consisting of vincristine, doxorubicin, etoposide, ifosfamide, and cyclophosphamide. A 12-yearold girl received a two-drug therapy using a combination of ifosfamide and doxorubicin. All three patients are in remission following chemotherapy and surgery, confirming the effectiveness and safety of the outlined regimens. There is a lack of consensus regarding an appropriate therapy algorithm for Ewing sarcoma patients with BCOR. The present study adds to the extant literature by detailing effective, yet varying treatment modalities.https://doi.org/10.15264/cpho.2022.29.2.60bcor sarcomabcor-ccnb3 fusionchemotherapypediatric oncology |
| spellingShingle | Salma Omar Karen Albritton Kenneth Heym Jason Wang Anish Ray Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case Series Clinical Pediatric Hematology-Oncology bcor sarcoma bcor-ccnb3 fusion chemotherapy pediatric oncology |
| title | Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case Series |
| title_full | Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case Series |
| title_fullStr | Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case Series |
| title_full_unstemmed | Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case Series |
| title_short | Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case Series |
| title_sort | multimodal treatment of sarcomas linked to bcor ccnb3 fusion in pediatrics a 3 patient case series |
| topic | bcor sarcoma bcor-ccnb3 fusion chemotherapy pediatric oncology |
| url | https://doi.org/10.15264/cpho.2022.29.2.60 |
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